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A Study to Determine the Efficacy and Safety of Luspatercept in Adult Participants and to Evaluate the Safety and Pharmacokinetics in and Adolescent Participants With Alpha (α)-Thalassemia

Phase 2
Recruiting
Conditions
Anemia
Interventions
Registration Number
NCT05664737
Lead Sponsor
Bristol-Myers Squibb
Brief Summary

The purpose of the study is to evaluate the efficacy and safety of luspatercept plus best supportive care (BSC) vs placebo plus BSC on anemia in adult participants with α-thalassemia hemoglobin H (HbH) disease and determine the safety and drug levels in adolescent participants.

Detailed Description

Not available

Recruitment & Eligibility

Status
RECRUITING
Sex
All
Target Recruitment
249
Inclusion Criteria
  • Adult participant≥ 18 years with documented diagnosis of A-Thal HbH disease with Transfusion dependence defined as:.

    1. TD participant: ≥ 6 RBC units during the 24 weeks prior to randomization.
    2. NTD participant:< 6 RBC units during the 24 weeks prior to randomization(transfusion due to conditions other than A-Thal will not be considered)and, RBC transfusion-free during at least 8 weeks prior to randomization(unless transfusion was required to treat an acute medical condition other than A-Thal) and, mean baseline Hb ≤ 10 g/dL, based on a minimum of 2 measurements ≥ 1 week apart within 4 weeks prior to randomization; hemoglobin values within 21 days post-transfusion will be excluded.
  • Adult participant has Eastern Cooperative Oncology Group (ECOG) 34 score of 0 or 1.

  • Adolescent participant 12 years to < 18 years with documented diagnosis of A-Thal HbH disease with transfusion dependence defined as:.

    1. TD participant: ≥ 4 RBC events during the 24 weeks prior to enrollment and, no transfusion-free period for > 56 days during the 24 weeks prior to enrollment. Participants must have a history of regular transfusions for at least 2 years.
    2. NTD participant:< 4 RBC events during the 24 weeks prior to enrollment and RBC transfusion-free during at least 8 weeks prior to enrollment and, mean baseline Hb ≤ 10 g/dL, based on a minimum of 2 measurements ≥ 1 week apart within 4 weeks prior to enrollment, hemoglobin values within 21 days post-transfusion will be excluded.
    3. Participant has Karnofsky (age ≥16 years) or Lansky (age < 16 years) performance status score ≥ 50 at screening.

Key

Exclusion Criteria
  • Medical Conditions: Diagnosis of A-ThalTrait, Hb Bart hydrops, ATRx A-Thal, hemoglobin S/β-thalassemia, myelodysplasia subtype anemia, or with HbE homozygous beta gene mutation. Anemia related to nutritional deficiency, anemia of chronic disease, autoimmune hemolytic anemia, or any other hemolytic anemias. Undergone episodes of hemolysis not related to A-Thal within the 8 weeks prior to randomization.
  • Participant has deep vein thrombosis (DVT), stroke or other thromboembolic event(s) (except clogged indwelling catheter) requiring medical intervention ≤ 24weeks prior to randomization.
  • Participant has uncontrolled hypertension. Controlled hypertension for this protocol is considered: blood pressure value corresponding to ≤Grade 1 according to NCI CTCAE Version 5.0. with or without pharmacological treatment.
  • Reproductive Status: Women who are pregnant, plan to get pregnant during the study, or who are breastfeeding.
  • Prior/Concomitant: Undergone HSCTs or gene therapy (candidates for HSCT or gene therapy with waiting period of ≥ 12 months are eligible).
  • Use of hydroxyurea treatment ≤ 12 weeks prior to enrollment for NTD participants and ≤ 24 weeks for TD participants.
  • Participant who has extramedullary hematopoiesis (EMH) complications requiring treatment to control the growth of EMH mass(es) during the screening period.
  • Any medical or psychiatric condition (including active infections, recent surgery, sequelae of diseases or interventions, clinically significant laboratory abnormalities or concurrent treatment) that in the opinion of the investigator would put the participant at unacceptable risk of participating in the study or that could affect interpretability of data.
  • Other protocol-defined inclusion/exclusion criteria apply.

Study & Design

Study Type
INTERVENTIONAL
Study Design
PARALLEL
Arm && Interventions
GroupInterventionDescription
Transfusion Dependent (TD): Luspatercept + Best supportive care (BSC)Luspatercept-
Adult TD Cohort: Placebo + BSCPlacebo-
Adult NTD Cohort: Placebo + BSCPlacebo-
Non-transfusion Dependent (NTD): Luspatercept + BSCLuspatercept-
Primary Outcome Measures
NameTimeMethod
Number of participants with ≥ 50% reduction from baseline in RBC transfusion burden with a reduction of at least 2 units during any continuous 12 weeks during Week 13-48 compared to 12-week interval immediately prior to date of first doseUp to Week 48

Adult TD Cohort

Dose-limiting toxicities (DLTs) defined as observance of ≥ Grade 3-related hemolytic crises or ≥ Grade 3-related event outside of the known safety profile occurring within the 21 days from their first dose of study therapyUp to Week 3

Adolescent TD and NTD Cohorts

Number of participants with an increase from baseline of ≥ 1.0 grams (g)/decilitre (dL) in mean hemoglobin (Hb) values over the continuous 12-week interval from Week 13 to Week 24 in the absence of RBC transfusionUp to Week 24

Adult NTD Cohort

Number of participants with adverse events (AEs)Up to 8.5 years

Adolescent TD and NTD Cohorts

Pharmacokinetics (PK): Serum concentration of LuspaterceptUp to Week 102

Adolescent TD and NTD Cohorts

Secondary Outcome Measures
NameTimeMethod
Number of participants with antidrug antibody (ADA)Up to Week 48

Adolescent TD and NTD Cohorts

Number of participants with ≥ 33% reduction from baseline in RBC transfusion burden with a reduction of at least 2 units during any continuous 24-week interval on treatment compared to 24-week interval immediately prior to date of first doseUp to Week 108

Adult TD Cohort

The longest duration with ≥ 50% reduction from baseline in RBC transfusion burden with a reduction of at least 2 unitsUp to Week 108

Adult TD Cohort

Number of RBC transfusion units from week 1 to week 48Up to Week 48

Adult and Adolescent TD Cohorts

Change from baseline in hemoglobin in the absence of transfusion at Week 24Up to Week 24

Adult and Adolescent NTD Cohorts

The longest duration of an increase from baseline of ≥ 1.0 g/dL in mean hemoglobin values starting from Week 13 in the absence of transfusionUp to Week 108

Adult NTD Cohort

Time Duration with an increase from baseline of ≥ 1.0 g/dL in hemoglobin values in the absence of transfusion within 48 weeksUp to Week 48

Adult NTD Cohort

Number of participants with an increase from baseline of ≥1.0 g/dL in mean Hb values over the continuous 12- week interval in the absence of transfusionUp to Week 24

Adult NTD Cohort

≥ 3 Increase from Baseline in Functional Assessment of Cancer Therapy Anemia Fatigue Subscale (FACT-An FS) Score from Baseline to the period from Week 13 to Week 24Up to Week 24

Adult NTD Cohort

Number of participants with AEsUp to 5 years

Adult and Adolescent TD and NTD Cohorts

Number of participants with laboratory abnormalitiesUp to 5 Years

Adult TD and NTD Cohorts

Number of participants with immunogenicityUp to 5 Years

Adult TD and NTD Cohorts

Number of participants with ≥ 50% reduction from baseline in RBC transfusion burden during any continuous 24-week interval within 48 weeks compared to the 24-week interval immediately prior to the date of first doseUp to Week 48

Adult TD Cohort

Number of participants with ≥ 33% reduction from baseline in RBC transfusion burden during any continuous 12-week interval compared to the 12-week interval immediately prior to the date of first doseUp to Week 108

Adult TD Cohort

Number of participants with ≥ 33% reduction from baseline in RBC transfusion burden from Week 13 to Week 24 and Week 37 to Week 48 compared to the 12-week interval immediately prior to the date of first doseUp to Week 48

Adult TD Cohort

Number of participants with ≥ 33% reduction from baseline in RBC transfusion burden from Week 1 to Week 24 and Week 25 to Week 48 compared to the 24-week interval immediately prior to the date of first doseUp to Week 48

Adult TD Cohort

Number of participants with ≥ 50% reduction from baseline in RBC transfusion burden from Week 13 to Week 24 and Week 37 to Week 48 compared to the 12-week interval immediately prior to the date of first doseUp to Week 48

Adult TD Cohort

Number of participants with ≥ 50% reduction from baseline in RBC transfusion burden from Week 1 to Week 24 and Week 25 to Week 48 compared to the 24-week interval immediately prior to the date of first doseUp to Week 48

Adult TD Cohort

Change from baseline in total RBC units transfused from Week 1 to Week 24, Week 25 to Week 48, and Week 1 to Week 48Up to Week 48

Adult TD Cohort

The longest duration of RBC transfusion-free period for participants who achieve transfusion-free period of ≥ 12 weeksUp to Week 108

Adult TD Cohort

The longest duration of reduction in transfusion burden for participants who achieve a response (rolling 12-week and 24-week response, both for ≥ 33% and ≥ 50% reduction)Up to Week 108

Adult TD Cohort

Time from first dose to first day of response (rolling 12-week and 24-week response, both for ≥ 33% and ≥ 50% reduction)Up to Week 108

Adult TD Cohort

Change from baseline in number of transfusion events at Week 48Up to Week 48

Adult TD Cohort

Number of participants who achieve RBC transfusion-free period of any continuous ≥ 12 weeks during treatmentUp to Week 108

Adult and Adolescent TD and NTD Cohorts

Number of participants who achieve RBC transfusion-free period of any continuous ≥ 24 weeks during treatmentUp to Week 108

Adult and Adolescent TD and NTD Cohorts

Time to first transfusionUp to Week 108

Adult and Adolescent NTD Cohorts

Number of transfusionsWithin 48 Weeks

Adult NTD Cohort

Number of transfusion visits/unitsWithin 48 Weeks

Adult NTD Cohort

Change from baseline in mean hemoglobin values over the continuous 12-week interval from Week 13 to Week 24 and Week 37 to Week 48 in the absence of transfusionsUp to Week 48

Adult TD and NTD Cohorts

Number of participants achieving an increase from baseline of ≥1.0g/dL or ≥1.5g/dL in mean Hb values in absence of transfusions from Week 13 to Week 24, Week 37 to Week 48 and during any continuous 12-week window within 24 weeks and 48 weeksUp to Week 48

Adult NTD Cohort

Time from first to last Hb measurement with increase from baseline by ≥ 1.0 g/dLUp to Week 108

Adult NTD Cohorts

Time to the first increase from baseline of ≥ 1.0 g/dL in mean Hb valueUp to Week 48

Adolescent NTD Cohort

Number of participants who achieve an increase in mean Hb of >10g/dL values during any continuous 12-week and 24-week interval within 48 weeks in the absence of transfusionsUp to Week 48

Adult NTD Cohort

Change from baseline in self-reported health-related quality of life (HRQoL) assessed by physical component summary (PCS) and mental component summary (MCS) of 36-item short-form health survey version2 (SF-36v2) at Week 24 and Week 48Up to Week 48

Adult TD and NTD Cohorts

Change from baseline in non-transfusion dependent β-thalassemia patient-reported outcome (NTDT-PRO) Tiredness/weakness (T/W) and shortness of breath (SoB) domain scores from Week 13 to Week 24 and from Week 37 to Week 48Up to Week 48

Adult NTD Cohort

Change from baseline in FACT-An FS Score at Week 24 and Week 48Up to Week 48

Adult NTD Cohort

Change from baseline in Functional Assessment of Cancer Therapy Anemia Anemia Subscale (FACT-An AS) at Week 24 and Week 48Up to Week 48

Adult NTD Cohort

Number of participants with at least one hemolytic crisisUp to Week 108

Adult TD and NTD Cohorts

Rate of hemolytic crisesUp to Week 108

Adult TD and NTD Cohorts

Time to first hemolytic crisisUp to Week 108

Adult TD and NTD Cohorts

Time to second hemolytic crisisUp to Week 108

Adult TD and NTD Cohorts

Change from baseline in hemolysis markers at Week 24 and Week 48Up to Week 48

Adult TD and NTD Cohorts:

Change from baseline in the 6-minute walk test (6MWT) distance at Week 24 and Week 48Up to Week 48

Adult NTD Cohort

Pharmacokinetics (PK): Serum concentration of LuspaterceptUp to Week 108

Adult and Adolescent TD and NTD Cohorts

Percent Change from Baseline in Biomarkers for Erythropoiesis at Week 84Baseline, Week 84

Adult TD and NTD Cohorts

The biomarkers for erythropoiesis to be evaluated include Hb variants including hemoglobin H (HbH), sTfR1, erythropoietin (EPO), growth differentiation factor (GDF11), GDF8, GDF15. The change will be measured as a percentage of change from baseline for all the biomarkers.

Percent Change from Baseline in Biomarkers and Parameters for Iron Homeostasis at Week 84Baseline, Week 84

Adult TD and NTD Cohorts

The biomarkers and parameters for iron homeostasis to be evaluated include hepcidin, erythroferrone (ERFE), serum ferritin, liver iron concentration (LIC), myocardial iron, iron chelation therapy (ICT).

The change will be measured as a percentage of change from baseline for all the biomarkers.

Change in mean corpuscular volume (MCV) at Week 48Baseline, Week 48

Adult TD and NTD Cohorts

Change in mean corpuscular hemoglobin (MCH) at Week 48Baseline, Week 48

Adult TD and NTD Cohorts

Change in nucleated red blood cells (nRBC) at Week 48Baseline, Week 48

Adult TD and NTD Cohorts

Change in red blood cells (RBC) at Week 48Baseline, Week 48

Adult TD and NTD Cohorts

The longest duration with reduction from baseline in the RBC transfusion burdenUp to Week 48

Adolescent TD Cohort

The number of participants with ≥ 50% reduction from baseline in RBC transfusion burden during an continuous 12 weeks during Weeks 13-48Up to Week 48

Adolescent TD Cohort

The number of participants with ≥ 33% reduction from baseline in RBC transfusion burden during an continuous 24 weeksUp to Week 48

Adolescent TD Cohort

Number of participants achieving an increase from baseline of ≥1.0g/dL in mean Hb values in absence of transfusions from Week 13 to Week 24Up to Week 24

Adolescent NTD Cohort

Cumulative time (in weeks) with an increase from baseline of ≥1.0g/dL in mean Hb values in absence of RBC transfusions within 48 weeksUp to Week 48

Adolescent NTD Cohort

Number of participants who achieve an increase in mean Hb of >10g/dL values during any continuous 12-week interval during week 13 to week 48 in the absence of transfusionsUp to Week 48

Adolescent NTD Cohort

The longest duration with an increase from baseline of ≥1.0g/dL in mean Hb values in absence of transfusionsUp to Week 48

Adolescent NTD Cohort

Mean change in biomarkers for hemolysisUp to Week 48

Adolescent TD and NTD Cohorts

Biomarkers for hemolysis to be evaluated include total/direct/indirect bilirubin, serum lactate dehydrogenase (sLDH), haptoglobin, reticulocytes and nucleated red blood cells, reticulocyte production index (RPI), and urinary urobilinogen

Mean change in biomarkers and parameters for iron homeostasisUp to 1 Year

Adolescent TD and NTD Cohorts

The biomarkers and parameters for iron homeostasis to be evaluated include serum ferritin, LIC, myocardial iron concentration (MIC), ICT, myocardial T2(TD participants), and extramedullary hematopoiesis (EMH) mass(es) when present (NTD participants)

Hematologic assessmentsUp to Week 48

Adolescent TD and NTD Cohorts

The hematologic assessments to be evaluated are red blood cell count, hemoglobin, hematocrit, reticulocyte count, nucleated red blood cell count, platelet count, mean cell volume, mean cell hemoglobin, mean corpuscular hemoglobin concentration, red blood cell distribution width, red blood cell morphology, and globin precipitates

The change from baseline in the number of health care resource utilization (HCRU)Up to Week 48

Adolescent TD and NTD Cohorts

Mean change from baseline in Pediatric Quality of Life Inventory (PedsQL) domain scoresUp to Week 48

Adolescent TD and NTD Cohorts

Mean change from baseline EQ-5D-5L utility indexUp to Week 48

Adolescent TD and NTD Cohorts

Mean change from baseline visual analogue scale (VAS) scoresUp to Week 48

Adolescent TD and NTD Cohorts

Trial Locations

Locations (36)

Local Institution - 0008

🇨🇦

Halifax, Nova Scotia, Canada

The First People's Hospital of Foshan

🇨🇳

Foshan, Guangdong, China

Local Institution - 0029

🇨🇳

Guangzhou, Guangdong, China

Nanfang Hospital of Southern Medical University

🇨🇳

Guangzhou, Guangdong, China

Maoming People's Hospital

🇨🇳

Maoming Shi, Guangdong, China

Local Institution - 0030

🇨🇳

Shenzhen Shi, Guangdong, China

Liuzhou People's Hospital

🇨🇳

Liuzhou, Guangxi, China

People's Liberation Army The 923rd Hospital

🇨🇳

Nanning Shi, Guangxi, China

Hainan Medical College - First Affiliated Hospital

🇨🇳

Haikou, Hainan, China

First People's Hospital of Yunnan Province

🇨🇳

Kunming, Yunnan, China

Hainan General Hospital

🇨🇳

Haikou, China

The First Affiliated Hospital of Guangxi Medical University

🇨🇳

Nanning, China

Local Institution - 0005

🇬🇷

Thessaloniki, B, Greece

Local Institution - 0007

🇬🇷

Larissa, E, Greece

Local Institution - 0018

🇬🇷

Rio, G, Greece

Local Institution - 0006

🇬🇷

Athens, Greece

Local Institution - 0009

🇬🇷

Goudi, Greece

Queen Mary Hospital

🇭🇰

Hong Kong, HK, Hong Kong

Local Institution - 0024

🇭🇰

Hong Kong Island, Hong Kong

Local Institution - 0022

🇮🇹

Cagliari, CA, Italy

Local Institution - 0026

🇮🇹

Genova, GE, Italy

Local Institution - 0020

🇮🇹

Orbassano, TO, Italy

Local Institution - 0028

🇮🇹

Napoli, Italy

Local Institution - 0019

🇮🇹

Napoli, Italy

Local Institution - 0033

🇲🇾

Johor Bahru, Malaysia

Local Institution - 0032

🇲🇾

Kuala Lumpur, Malaysia

Local Institution - 0035

🇸🇦

Al-Ahsa, Saudi Arabia

Local Institution - 0034

🇸🇦

Riyadh, Saudi Arabia

Local Institution - 0036

🇸🇬

Singapore, Singapore

Kaohsiung Medical University Chung-Ho Memorial Hospital

🇨🇳

Kaohsiung, KHH, Taiwan

National Taiwan University Hospital

🇨🇳

Nan Gang Qu, TPE, Taiwan

China Medical University Hospital

🇨🇳

Taichung, TXG, Taiwan

Siriraj Hospital

🇹🇭

Bangkok, Thailand

Local Institution - 0031

🇹🇭

Mueang Phitsanulok, Thailand

Local Institution - 0027

🇹🇷

Altındağ, Turkey

Local Institution - 0021

🇹🇷

Topkapı, Turkey

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